Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection

Background-We sought to determine era-specific changes in the incidence of mortality and reoperation in children with total anomalous pulmonary venous connection. Methods and Results-We reviewed the records of 377 children presenting from 1946 to 2005 with total anomalous pulmonary venous connection. Multivariable parametric regression models determined the incidence and risk factors for death and reoperation after repair. Pulmonary venous connection was supracardiac in 44%, infracardiac in 26%, cardiac in 21%, and mixed in 9%. Pulmonary venous obstruction was present in 48% at presentation, most frequently with infracardiac connection type (P < 0.001). In total, 327 patients were repaired (median age, 1.7 months). Overall survival from repair was 65 +/- 6% at 14 years, with a current survival of 97%. Significant (P < 0.01) incremental risk factors for postrepair death were cardiac connection type, earlier operation year, younger age at repair, use of epinephrine postoperatively, and postoperative pulmonary venous obstruction. More recent operation year was associated with younger age at repair (P < 0.001), decreased use of deep hypothermic circulatory arrest (P < 0.001), and use of specific drugs postoperatively (P < 0.001). Risk-adjusted estimated 1-year survival for a patient repaired at birth with unfavorable morphology in 2005 is 37% (95% CI, 8 to 80) compared with 96% (95% CI, 91 to 99) for a patient with favorable morphology repaired at 1 year of age. Freedom from reoperation was 82 +/- 6% at 11 years after repair, with increased risk associated with mixed connection type (P = 0.04) and postoperative pulmonary venous obstruction (P < 0.001). Conclusions-Mortality after total anomalous pulmonary venous connection repair has decreased but remains highest in young patients and in those with cardiac connection type or pulmonary venous obstruction. Unfavorable anatomic characteristics remain important determinants of postrepair survival despite improved perioperative care.